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4 results

  • Eosinophilic granulomatosis with polyangiitis and laryngeal involvement: review of the literature and a cross-sectional prospective experience

  • V Seccia, L Cristofani-Mencacci, I Dallan, S Fortunato, M L Bartoli, S Sellari-Franceschini, M Latorre, P L Paggiaro, C Baldini
  • Journal:
    The Journal of Laryngology & Otology / Volume 132 / Issue 7 / July 2018
    Published online by Cambridge University Press:
    11 June 2018, pp. 619-623
    Print publication:
    July 2018
  • Background

    Eosinophilic granulomatosis with polyangiitis and granulomatosis with polyangiitis show variable otorhinolaryngological involvement. Up to 14 per cent of granulomatosis with polyangiitis patients have subglottis involvement; little is known about the laryngeal involvement in eosinophilic granulomatosis with polyangiitis.

    Method

    A literature review was conducted, together with a prospective cross-sectional analysis of 43 eosinophilic granulomatosis with polyangiitis patients. All patients underwent fibre-optic laryngoscopy with narrow-band imaging, and completed health-related questionnaires.

    Results

    The literature review showed only two cases of laryngeal involvement in eosinophilic granulomatosis with polyangiitis; in our cohort, no cases of subglottis stenosis were found, but local signs of laryngeal inflammation were present in 72 per cent of cases. Of the patients, 16.2 per cent had a pathological Reflux Finding Score (of 7 or higher).

    Conclusion

    Laryngeal inflammation in eosinophilic granulomatosis with polyangiitis is frequent. It is possibly due more to local factors than to eosinophilic granulomatosis with polyangiitis itself. However, ENT evaluation is needed to rule out possible subglottis inflammation. These findings are in line with current literature and worthy of confirmation in larger cohorts.

  • 55 - EPIDERMAL NEVUS SYNDROME

  • from PART V: - SYSTEMIC DISORDERS THAT ALSO INVOLVE THE CEREBROVASCULAR SYSTEM
  • Edited by Louis R. Caplan, Julien Bogousslavsky
  • Book:
    Uncommon Causes of Stroke
    Published online:
    06 January 2010
    Print publication:
    09 October 2008, pp 401-404

  • Summary

    The Churg-Strauss syndrome (CSS) is a type of small-vessel vasculitis that involves the capillaries, arterioles, and venules. There are different clinical and histopathological criteria for the diagnosis of CSS. The clinical criteria of J. G. Lanham and his group for the diagnosis of CSS is a triad consisting of asthma, peak eosinophilia > 1.5 x 109/L, and systemic vasculitis involving two or more extrapulmonary organs. Glucocorticoids remain the main stay of therapy in CSS. Initial therapy with prednisone is usually pulsed (15 mg/kg over 60 minutes repeated at 24-hour intervals for 1-3 days) followed by prednisone 1 mg/kg per day. Involvement of the central nervous system (CNS) is associated with an increased risk for mortality and requires aggressive treatment with steroids and immunosuppressants. The condition should be considered in any patient who has asthma and develops eosinophilia and peripheral and/or CNS signs.

  • Unusual presentation of temporal bone involvement in Churg–Strauss syndrome

  • M Martinez Del Pero, D Moffat, H Sudhoff
  • Journal:
    The Journal of Laryngology & Otology / Volume 122 / Issue 4 / April 2008
    Published online by Cambridge University Press:
    10 April 2007, pp. 425-427
    Print publication:
    April 2008
  • Objective:

    To present a case of a 60-year-old male with a history of sudden onset sensorineural hearing loss due to Churg–Strauss syndrome.

    Case report:

    The patient had a 20-year history of asthma and recurrent right otitis media and a nasal polypectomy four years prior to presenting with ear symptoms. Ear, nose and throat involvement is common in Churg–Strauss syndrome, usually manifesting as allergic rhinitis and chronic rhinosinusitis with or without polyps.

    Conclusions:

    Otolaryngologists play an important role in making an early diagnosis of this disease. To our knowledge this is the first case of Churg–Strauss syndrome primarily presenting with otological pathology: left sensorineural hearing loss and right otitis media.